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ANSWER

Aortic coarctation: The patient’s hypertension and murmur are likely caused by a previously undiagnosed
coarctation of the aorta. The chest radiograph demonstrates an enlarged collateral intercostal arterial
circulation that has caused notching of the inferior-posterior rib margins, which is supportive of the
diagnosis. Magnetic resonance angiography (MRA) confirms the presence of a postductal aortic
coarctation (Image 2).

Coarctation of the aorta is a congenital condition that results in narrowing of a segment of the aorta. It was first
described by Giovanni Morgagni in 1760. Historically, coarctation of the aorta was classified as preductal (before
the origin of the ductus arteriosus) or juxtaductal (distal to the origin of the ductus arteriosus). More recently, the
latter designation has been abandoned for the term postductal. The postductal form is the most common type of
coarctation of the aorta. Coarctation of the aorta constitutes approximately 6-8% of all congenital heart diseases in
infants. It is most frequently associated with other forms of congenital heart disease, such as ventricular septal
defects, patent ductus arteriosus, bicuspid aortic valves, and aortic stenoses; however, it may also appear as an
isolated condition. It is more common in males than in females; in patients with ovarian agenesis (Turner
syndrome), it is particularly common.

Patients diagnosed with coarctation of the aorta in infancy usually have a combination of other heart anomalies that
can lead to overt congestive heart failure. Those patients presenting beyond infancy, such as in this case, often have
vague symptoms that may include headaches, a propensity to nose bleeds, leg cramps, and cold feet. Hypertension
is usually present and leads to further in-depth testing. The hallmark physical sign of postductal coarctation is that
blood pressure in the arms is at least 20 mm Hg higher than it is in the lower extremities (normally blood pressure in
the lower extremities is slightly higher than in the upper extremities). A systolic or continuous murmur in the
infrascapular or infraclavicular area also may suggest the presence of this anomaly. Additional murmurs may
indicate the presence of associated anomalies, such as ventricular septal defects, patent ductus arteriosus, or aortic
stenoses.

Chest radiography may demonstrate cardiomegaly and rib notching from the compensatory collateral intercostal
arterial dilatation. The classic “figure 3” sign seen on chest radiographs occurs in at least one third of patients, and
results from prestenotic dilatation of the ascending aorta followed by indentation of the aorta at the coarctation site
and poststenotic dilatation of the descending aorta. The “reverse 3” or “E” sign can also be observed on barium
swallow studies, resulting from matched compression of the esophagus by the dilated segments of the aorta. Signs
of left ventricular hypertrophy are also often seen on electrocardiograms (ECGs). An echocardiogram is usually
performed to detect any associated cardiac anomalies. MRA and cardiac catheterization may be necessary to
confirm the exact location and the presence of collaterals. A gradient of more than 20% across the stenosis during
cardiac catheterization indicates a severe coarctation and requires urgent intervention.

Treatment of aortic coarctation is usually surgical. Antihypertensive medication must be used with caution, as its use
may lead to inadequate perfusion of the lower body and renal impairment. The best treatment strategy is admission
for early repair. If left untreated, 90% of patients with aortic coarctation die by the age of 50 years, usually from
hypertensive complications. Historically, surgical resection of the involved aortic segment has been the treatment of
choice, and it has a high success rate. More recently, balloon dilatation and placement of endovascular stents are
gaining popularity and becoming accepted forms of primary therapy, especially for patients who may be unsuitable
candidates for surgery. Postoperative complications may be acute or delayed and include persistent hypertension,
recoarctation, and aortic aneurysms, among others.

This patient had an echocardiogram that failed to reveal any associated cardiac defects. He was referred for surgical
intervention and underwent successful resection of the coarcted aortic segment after resolution of his associated
pancreatitis, the initial reason for his presentation and admission.

For more information on aortic coarctation, please visit the eMedicine articles Aortic Coarctation, in the Medicine,
Ob/Gyn, Psychiatry, and Surgery section; Aorta, Coarctation, in the Radiology section; and Coarctation of the
Aorta, in the Pediatrics section.

References:

1. Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown) 2007 Feb;
8(2): 123-8 [MEDLINE: 17299295]
2. Beekman RH, Riemanschneider TA, Emmanouilides GC. Coarctation of the aorta. In: Moss and Adams’
Heart Disease in Infants, Children and Adolescents. 5th ed. Baltimore, Md: Lippincott Williams &
Wilkins; 1995: 1111-33
3. Shah S, Calderon DM. Aortic coarctation. eMedicine Journal [serial online]. Last updated: Jun 23, 2005.
Available at: http://www.emedicine.com/MED/topic154.htm. Date accessed: 8/23/07.
CASE DIAGNOSIS
What is the diagnosis?
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HINT
The next step should be to check the blood pressure in the lower extremities.
Authors:
Girendra Hoskere, MD, Fellow, Division of Pulmonary Diseases and Critical Care Medicine, James H. Quillen
College of Medicine, East Tennessee State University

Ryland P. Byrd, Jr, MD, Chief of Pulmonary Medicine, Medical Director of Respiratory Therapy, Quillen
Mountain Home Veterans Affairs Medical Center, Professor, Department of Internal Medicine, Division of
Pulmonary Diseases and Critical Care Medicine, James H. Quillen College of Medicine, East Tennessee State
University

Thomas M. Roy, MD, Chief of the Division of Pulmonary Diseases and Critical Care Medicine and Professor of
Medicine Department of Internal Medicine, James H. Quillen College of Medicine, East Tennessee State
University

eMedicine Editors:
Brady Pregerson, MD, Dept. of Emergency Medicine, Cedars-Sinai Medical Center, Los Angeles, CA; Dept. of
Emergency Medicine, Tri-City Medical Center, Oceanside, CA

Rick G. Kulkarni, MD, FACEP, Assistant Professor, Yale School of Medicine, Section of Emergency Medicine,
Department of Surgery, Attending Physician, Medical Director, Department of Emergency Services, Yale-New
Haven Hospital, CT

Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center, Seattle, Wash.